Jakowski Joseph D, Wakely Paul E
Department of Pathology, The Ohio State University College of Medicine, Columbus, OH 43210, USA.
Hum Pathol. 2008 Apr;39(4):623-8. doi: 10.1016/j.humpath.2007.08.017. Epub 2008 Feb 14.
Low-grade fibromyxoid sarcoma (LGFMS) is a rare neoplasm commonly affecting young adults and typically arising in the somatic soft tissue of the proximal extremities. Its occurrence within the thoracic cavity is exceedingly rare. We report an LGFMS arising from the epicardial surface of the right side of the heart in a 44-year-old woman. Diagnosis was aided by a strikingly characteristic light microscopic appearance of a bland spindle cell sarcoma containing numerous so-called giant collagen rosettes and supported by immunohistology, evidence of FUS translocation by fluorescence in situ hybridization, and electron microscopy demonstrating a fibroblastic phenotype. Aspiration cytology showed a nonspecific bland spindle cell lesion. Review of the literature uncovered 5 previously reported examples of intrathoracic LGFMS. Low-grade fibromyxoid sarcoma is probably an underrecognized intrathoracic neoplasm and should be considered in the differential diagnosis of spindle cell neoplasms of the mediastinum, pleura, heart, and lungs.
低度纤维黏液样肉瘤（LGFMS）是一种罕见的肿瘤，常见于年轻人，通常发生于近端肢体的体壁软组织。其发生于胸腔内极为罕见。我们报告一例发生于一名44岁女性心脏右侧心外膜表面的LGFMS。诊断借助于具有显著特征的光镜表现，即一种含有众多所谓巨大胶原玫瑰花结的温和梭形细胞肉瘤，并得到免疫组织学、荧光原位杂交显示的FUS易位证据以及证明成纤维细胞表型的电镜检查的支持。细针穿刺细胞学显示为非特异性温和梭形细胞病变。文献复习发现5例先前报道的胸腔内LGFMS病例。低度纤维黏液样肉瘤可能是一种未被充分认识的胸腔内肿瘤，在纵隔、胸膜、心脏和肺部梭形细胞肿瘤的鉴别诊断中应予以考虑。
